Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis.

BACKGROUND: We aimed to compare neuropathic progression rate between hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) and other peripheral neuropathies, including diabetic peripheral neuropathy (DPN) and Charcot-Marie-Tooth disease (CMT). METHODS: Literature searches identified studies reporting neuropathic progression, measured by Neuropathy Impairment Score (NIS) or NIS-Lower Limbs (NIS-LL). Our study also included unpublished data from a clinical registry of patients who were diagnosed with different peripheral neuropathies and seen at the Oregon Health & Science University (OHSU) during 2016-2020. Meta-analysis and meta-regression models examined and compared annual progression rates, calculated from extracted data, between studies of ATTRv-PN and other peripheral neuropathies. RESULTS: Data were synthesized from 15 studies in which NIS and/or NIS-LL total scores were assessed at least twice, with ≥12 weeks between assessments, among untreated patients with ATTRv-PN or other peripheral neuropathies. Meta-analysis models yielded that the annual progression rate in NIS total scores was significantly different from zero for studies in ATTRv-PN and CMT (11.77 and 1.41; both P < 0.001), but not DPN (- 1.96; P = 0.147). Meta-regression models showed significantly faster annual progression in studies in ATTRv-PN, which statistically exceeded that in other peripheral neuropathies by 12.45 points/year for NIS, and 6.96 for NIS-LL (both P < 0.001). CONCLUSIONS: Peripheral nervous function deteriorates more rapidly in patients with ATTRv-PN than for other peripheral neuropathies. These findings may improve understanding of the natural history of neuropathy in ATTRv-PN, facilitate early diagnosis, and guide the development of assessment tools and therapies specifically targeting neuropathic progression in this debilitating disease.

Prospective Collection of PROMIS Physical Function Measure Demonstrates Significant Improvement After Bariatric Surgery.

BACKGROUND: We evaluated quality of life among bariatric surgery patients using patient-reported outcomes (PROs). We hypothesized that physical function would improve after bariatric surgery. METHODS: We prospectively collected PROs beginning in December 2015. We used the validated Patient-Reported Outcomes Measurement Information System (PROMIS) instruments because of their broad applicability and ability to use computer-adapted technology to survey. Measures are repeated at clinic visits, both pre- and postoperatively. Data were reviewed through February 2018. Data were analyzed comparing pre- and postop physical function PRO (PF PRO) by procedure: laparoscopic Roux-en-Y gastric bypass (LRYGB) or sleeve gastrectomy (LSG). Additional variables were included in an adjusted linear mixed-effects regression model in order to isolate the effect of surgery on PF PRO over time. RESULTS: This cohort included 279 bariatric surgery patients. The mean follow-up time was 1.5 years after surgery. The procedure groups were similar in terms of age and race but differed by gender and preoperative BMI. The PF-PRO measure showed significant improvement following surgery for both procedures. CONCLUSION: Patient-reported physical function improved significantly after bariatric surgery. There was no significant difference between procedures.